Jay Ingram, who excels at explaining science to Canadians, looks at new investigations of brain diseases.

Alzheimer’s, Parkinson’s, or the rarer Creutzfeldt-Jakob disease, are some of the neurodegenerative terrors that haunt boomers as they age. Little is known how these withering diseases develop, why some people come down with them while others don’t; if they can possibly pass from one species to another; if they are perhaps caused by a virus or infection.

Jay Ingram, whose métier is simplifying science for Canadians, examines new research into these devastating illnesses. His book, Fatal Flaws, How a Misfolded Protein Baffled Scientists And Changed The Way We Look at The Brain, explores the controversial science around a molecule called a prion. Discovered in the 1980s by scientist Stanley Prusiner, prions occur in mad cow disease. There is a possible link to Alzheimer’s and Parkinson’s, too. I talked to Ingram about Fatal Flaws which is being published this week.

Star: Can you explain what a prion is?

Ingram: It is an improperly folded version of a molecule found throughout our bodies. Proteins, which prions are, need to fold up in exactly the right shape to work. We have millions of different proteins in our bodies. A prion is one of those proteins very common in the brain that can get misfolded and when that happens it can cause others to get misfolded and you get a runaway effect.

Star: Why are they controversial?

Ingram: Nobody believed you could have something that didn’t have any genes. Bacteria have genes. Viruses have genes. A prion doesn’t have any genes, yet it can reproduce. It can kill you by reproducing in your brain.

Star: What’s the connection to Alzheimer’s and Parkinson’s?

Ingram: Alzheimer’s and Parkinson’s share similarities to prion diseases. You get these misfolded proteins that accumulate. They cause the plaque in Alzheimer’s, for example. But, unlike Creutzfeldt-Jakob disease, it has never been shown that Alzheimer’s is infectious. The brain disease, Chronic Traumatic Encephalopathy, that affects hockey players and football players who get head injuries also involves misfolded proteins.

Star: There is a human version of mad cow disease which killed 171 young Brits in the 1990s. We eat lamb. Why don’t people get the human version of sheeps’ scrapie? (Scrapie is a sheep illness likened to mad cow disease.)

Ingram: In a prion disease the misfolded prions have to be able to affect the normal ones and misfold them into an irregular shape. Maybe it’s how well they fit with each other that allows that to happen. So you could guess sheep scrapie prions don’t match very well with the normal one in humans whereas the mad cow one does. You are getting down to the molecular level and it is pretty challenging to understand.

Star: Do you think we are on the brink of understanding how Alzheimer’s and other neurodegenerative diseases occur and finding cures?

Ingram: I would be willing to bet the kind of understanding of misfolded proteins that has come from prion science will shed dramatic light on ALS, Parkinson’s and Alzheimer’s. There are others. Juvenile diabetes has misfolded proteins too. You have to figure out how a misfolded protein can cause another to create a misfolded shape and make it nasty.

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